SPTCL
MCID: TCL023
MIFTS: 38

T-Cell Lymphoma, Subcutaneous Panniculitis-Like (SPTCL)

Categories: Blood diseases, Cancer diseases, Genetic diseases, Immune diseases, Rare diseases, Skin diseases

Aliases & Classifications for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

MalaCards integrated aliases for T-Cell Lymphoma, Subcutaneous Panniculitis-Like:

Name: T-Cell Lymphoma, Subcutaneous Panniculitis-Like 58
Subcutaneous Panniculitis-Like T-Cell Lymphoma 54 60 6 17
Sptcl 58 54 60
Subcutaneous Panniculitic T-Cell Lymphoma 60

Characteristics:

Orphanet epidemiological data:

60
subcutaneous panniculitis-like t-cell lymphoma
Inheritance: Not applicable; Age of onset: All ages;

OMIM:

58
Inheritance:
autosomal recessive

Miscellaneous:
variable severity
variable age at onset (median 36 years)
immunosuppressive drugs and hematopoietic bone marrow transplant are effective treatments


Classifications:



External Ids:

OMIM 58 618398
ICD10 34 C86.3
MESH via Orphanet 46 C537503
ICD10 via Orphanet 35 C83.6
UMLS via Orphanet 75 C0522624
Orphanet 60 ORPHA86884

Summaries for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

NIH Rare Diseases : 54 Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare type of lymphoma that mainly affects the skin. Lymphomas are cancers that result from a type of white blood cell that grows out of control. In SPTCL, people develop multiple painless nodules in the fat layer under the skin and can these cause swelling. This is known as panniculitis. The cause of this type of cancer is unknown, but genetic factors may be involved. It can be diagnosed by a skin biopsy of the affected area. Treatment for SPTCL depends on the symptoms but often involves taking a steroid medication or a medication that lessens the immune response. Some people with more aggressive SPTCL may need chemotherapy. SPTCL can occur in children or adults, but is typically diagnosed in the 30s. For unknown reasons, it occurs more often in women than men.  SPTCL is a very slow-growing cancer and does not usually spread beyond the subcutaneous fat layer. About 20% of people with SPTCL will also develop another condition called hemophagocytic lymphohistiocytosis (HLH) in which the body makes too many immune cells. These immune cells attack other cells in the body and cause an enlarged spleen and a decreased level of blood cells in the body. 

MalaCards based summary : T-Cell Lymphoma, Subcutaneous Panniculitis-Like, also known as subcutaneous panniculitis-like t-cell lymphoma, is related to lymphoma and lupus erythematosus. An important gene associated with T-Cell Lymphoma, Subcutaneous Panniculitis-Like is HAVCR2 (Hepatitis A Virus Cellular Receptor 2). The drugs Prednisone and Ifosfamide have been mentioned in the context of this disorder. Affiliated tissues include t cells, skin and bone marrow, and related phenotypes are fever and fatigue

OMIM : 58 Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is an uncommon form of T-cell non-Hodgkin lymphoma in which cytotoxic CD8 (see 186910)+ T cells infiltrate adipose tissue forming subcutaneous nodules. Both children and adults can be affected, with a median age at diagnosis of 36 years and a female gender bias. Most patients have accompanying systemic features such as fever or flank pain. A subset (about 20%) of patients develop hemophagocytic lymphohistiocytosis (HLH), usually associated with CD8+ T cells rimming adipocytes in the bone marrow. An infectious agent is not identified, and the disorder is believed to result from improperly activated inflammation. Immunosuppressive therapy may be helpful; hematopoietic bone marrow transplantation is usually curative (summary by Gayden et al., 2018). For a general discussion of genetic heterogeneity of HLH, see HLH1 (267700). (618398)

Related Diseases for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

Graphical network of the top 20 diseases related to T-Cell Lymphoma, Subcutaneous Panniculitis-Like:



Diseases related to T-Cell Lymphoma, Subcutaneous Panniculitis-Like

Symptoms & Phenotypes for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

Human phenotypes related to T-Cell Lymphoma, Subcutaneous Panniculitis-Like:

60 (show all 11)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 fever 60 Frequent (79-30%)
2 fatigue 60 Frequent (79-30%)
3 weight loss 60 Frequent (79-30%)
4 skin ulcer 60 Frequent (79-30%)
5 abnormality of the coagulation cascade 60 Frequent (79-30%)
6 hepatosplenomegaly 60 Frequent (79-30%)
7 panniculitis 60 Very frequent (99-80%)
8 erythematous papule 60 Very frequent (99-80%)
9 erythematous plaque 60 Frequent (79-30%)
10 chills 60 Frequent (79-30%)
11 hemophagocytosis 60 Frequent (79-30%)

Symptoms via clinical synopsis from OMIM:

58
Metabolic Features:
fever

Hematology:
anemia (in some patients)
pancytopenia (in some patients)

Abdomen Spleen:
splenomegaly (in some patients)

Neoplasia:
t-cell non-hodgkin lymphoma, subcutaneous

Skin Nails Hair Skin:
panniculitis
erythematous plaques
hemophagocytosis (in some patients)
subcutaneous nodules, diffuse
atypical cytotoxic cd8+ t cells rimming adipocytes

Head And Neck Face:
facial swelling (in some patients)

Immunology:
autoimmunity (in some patients)
increased levels of inflammatory cytokines, including tnf-alpha, il2, and il1b
hemophagocytic lymphohistiocytosis (hlh) (in some patients)
atypical cytotoxic cd8+ t cells rimming adipocytes in bone marrow (in some patients)

Laboratory Abnormalities:
increased triglycerides (in patients with hlh)
hypofibrinogenemia (in patients with hlh)
hyperferritinemia (in patients with hlh)

Clinical features from OMIM:

618398

Drugs & Therapeutics for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

Drugs for T-Cell Lymphoma, Subcutaneous Panniculitis-Like (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show top 50) (show all 68)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Prednisone Approved, Vet_approved Phase 4,Phase 1,Phase 2 53-03-2 5865
2
Ifosfamide Approved Phase 4 3778-73-2 3690
3
Etoposide Approved Phase 4 33419-42-0 36462
4
Vincristine Approved, Investigational Phase 4,Phase 1,Phase 2 2068-78-2, 57-22-7 5978
5
Dexamethasone Approved, Investigational, Vet_approved Phase 4 50-02-2 5743
6
Epirubicin Approved Phase 4 56420-45-2 41867
7
Cyclophosphamide Approved, Investigational Phase 4,Phase 1,Phase 2 50-18-0, 6055-19-2 2907
8
Dexamethasone acetate Approved, Investigational, Vet_approved Phase 4 1177-87-3
9
Cisplatin Approved Phase 4 15663-27-1 2767 441203 84093
10
Gemcitabine Approved Phase 4 95058-81-4 60750
11
Doxorubicin Approved, Investigational Phase 4,Phase 1,Phase 2 23214-92-8 31703
12
Mechlorethamine Approved, Investigational Phase 4 51-75-2 4033
13
leucovorin Approved Phase 4 58-05-9 143 6006
14
Methotrexate Approved Phase 4 1959-05-2, 59-05-2 126941
15
Folic Acid Approved, Nutraceutical, Vet_approved Phase 4 59-30-3 6037
16
Doxil Approved June 1999 Phase 4,Phase 1,Phase 2 31703
17
Pirarubicin Investigational Phase 4 72496-41-4
18 Hormones Phase 4,Phase 1,Phase 2,Not Applicable
19 Anti-Bacterial Agents Phase 4,Phase 1,Phase 2
20 Antimitotic Agents Phase 4,Phase 1,Phase 2
21 Hormones, Hormone Substitutes, and Hormone Antagonists Phase 4,Phase 1,Phase 2
22 Alkylating Agents Phase 4,Phase 1,Phase 2
23 Topoisomerase Inhibitors Phase 4,Phase 1,Phase 2
24 Antineoplastic Agents, Hormonal Phase 4,Phase 1,Phase 2
25 Immunosuppressive Agents Phase 4,Phase 1,Phase 2
26 Antirheumatic Agents Phase 4,Phase 1,Phase 2
27 Anti-Inflammatory Agents Phase 4,Phase 1,Phase 2
28 glucocorticoids Phase 4,Phase 1,Phase 2
29 Etoposide phosphate Phase 4
30 Immunologic Factors Phase 4,Phase 1,Phase 2
31 Antibiotics, Antitubercular Phase 4,Phase 1,Phase 2
32 BB 1101 Phase 4
33 Antineoplastic Agents, Phytogenic Phase 4,Phase 1,Phase 2
34 Hormone Antagonists Phase 4,Phase 1,Phase 2
35
Isophosphamide mustard Phase 4 0
36 Antineoplastic Agents, Alkylating Phase 4,Phase 1,Phase 2
37 Vitamin B Complex Phase 4
38 Folate Phase 4
39 Dermatologic Agents Phase 4
40 Vitamin B9 Phase 4
41 Antimetabolites, Antineoplastic Phase 4
42 Antimetabolites Phase 4
43 Nucleic Acid Synthesis Inhibitors Phase 4
44 Folic Acid Antagonists Phase 4
45
Vorinostat Approved, Investigational Phase 1, Phase 2,Phase 2 149647-78-9 5311
46
Bortezomib Approved, Investigational Phase 2,Phase 1 179324-69-7 387447 93860
47 Histone Deacetylase Inhibitors Phase 1, Phase 2,Phase 2
48
rituximab Approved Phase 1 174722-31-7 10201696
49
Sirolimus Approved, Investigational Phase 1 53123-88-9 46835353 5284616 6436030
50
Everolimus Approved Phase 1 159351-69-6 70789204 6442177

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 CEOP/IVE/GDP Compared With CEOP as the First-line Therapy for Newly Diagnosed Adult Patients With PTCL Unknown status NCT02533700 Phase 4 CEOP/IVE/GDP chemotherapy regimen;CEOP chemotherapy regimen for 6 cycles
2 CTOP/ITE/MTX Compared With CHOP as the First-line Therapy for Newly Diagnosed Young Patients With T Cell Lymphoma Active, not recruiting NCT01746992 Phase 4 Cyclophosphamide 750mg/m2;Vincristine 1.4mg/m2;Doxorubicin 50mg/m2;prednisone 60mg/m2;ifosfamide 2000mg/m2;pirarubicin 50mg/m2;pirarubicin 25mg/m2;Etoposide phosphate 100mg/m2;methotrexate 1500mg/m2
3 SAHA + CHOP in Untreated T-cell Non-Hodgkin's Lymphoma Completed NCT00787527 Phase 1, Phase 2 Zolinza (vorinostat);Cyclophosphamide;Doxorubicin;Vincristine;Prednisone
4 Combination of Vorinostat and Bortezomib in Relapsed or Refractory T-Cell Non-Hodgkin's Lymphoma Terminated NCT00810576 Phase 2 Vorinostat;Bortezomib
5 Phase 1 Trial of Siplizumab and Dose-Adjusted EPOCH-Rituximab in T- and NK-Cell Lymphomas Active, not recruiting NCT01445535 Phase 1 EPOCH
6 Everolimus and Bortezomib in Treating Patients With Relapsed or Refractory Lymphoma Terminated NCT00671112 Phase 1 Bortezomib;Everolimus
7 Cholecalciferol in Improving Survival in Patients With Newly Diagnosed Cancer With Vitamin D Insufficiency Recruiting NCT01787409 Not Applicable
8 Salvia Hispanica Seed in Reducing Risk of Disease Recurrence in Patients With Non-Hodgkin Lymphoma Active, not recruiting NCT02652715 Not Applicable

Search NIH Clinical Center for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

Genetic Tests for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

Anatomical Context for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

MalaCards organs/tissues related to T-Cell Lymphoma, Subcutaneous Panniculitis-Like:

42
T Cells, Skin, Bone Marrow, Adipocyte, Spleen, Bone, Nk Cells

Publications for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

Articles related to T-Cell Lymphoma, Subcutaneous Panniculitis-Like:

(show top 50) (show all 186)
# Title Authors Year
1
Subcutaneous Panniculitis-Like T-Cell Lymphoma With Granulomas as the Predominant Feature. ( 30908292 )
2019
2
Subcutaneous panniculitis-like T-cell lymphoma in a 25-year-old male patient with sickle cell disease. ( 30944066 )
2019
3
Epidemiology of primary cutaneous gamma/delta T-cell lymphoma and subcutaneous panniculitis-like T-cell lymphoma in the United States from 2006-2015: A Surveillance, Epidemiology, and End Results-18 analysis. ( 30951189 )
2019
4
Cutaneous mantle cell lymphoma histomorphologically mimicking subcutaneous panniculitis-like T-cell lymphoma: Case report. ( 30957249 )
2019
5
Successful treatment of a rare subcutaneous panniculitis-like T-cell lymphoma: An unusual case report and literature review. ( 30884048 )
2019
6
Frequent germline mutations of HAVCR2 in sporadic subcutaneous panniculitis-like T-cell lymphoma. ( 30792187 )
2019
7
Treatment of Facial Lipoatrophy Secondary to Subcutaneous Panniculitis-Like T-Cell Lymphoma. ( 30829756 )
2019
8
Pediatric Subcutaneous Panniculitis-like T-Cell Lymphoma With Hemophagocytosis Showing Complete Resolution With the BFM90 Protocol: Case Report and Review of Literature. ( 30730380 )
2019
9
Clinicopathologic, immunophenotypic, and molecular analysis of subcutaneous panniculitis-like T-cell lymphoma: A retrospective study in a tertiary care center. ( 30350476 )
2019
10
Composite cutaneous lymphoma of diffuse large B-cell lymphoma-leg type and subcutaneous panniculitis-like T-cell lymphoma. ( 29851123 )
2018
11
Hematopoietic stem cell transplantation for subcutaneous panniculitis-like T-cell lymphoma: single center experience in an Asian population. ( 30547418 )
2018
12
Hemophagocytic lymphohistiocytosis: A rare, potentially fatal complication in subcutaneous panniculitis like T cell lymphoma. ( 30289114 )
2018
13
The coexistence of lupus erythematosus panniculitis and subcutaneous panniculitis-like T-cell lymphoma in the same patient. ( 29892661 )
2018
14
Subcutaneous Panniculitis-Like T-Cell Lymphoma Versus Lupus Erythematosus Panniculitis: Distinction by Means of the Periadipocytic Cell Proliferation Index. ( 29742552 )
2018
15
Subcutaneous panniculitis-like T-cell lymphoma responsive to combination therapy with methotrexate and corticosteroids. ( 30677832 )
2018
16
Relapsed subcutaneous panniculitis-like T cell lymphoma evaluated by FDG PET/CT: A clinical case report. ( 30431571 )
2018
17
Subcutaneous panniculitis-like T-cell lymphoma: Clinical features, therapeutic approach, and outcome in a case series of 16 patients. ( 30126736 )
2018
18
A Case of Refractory Subcutaneous Panniculitis-like T-cell Lymphoma with Bone Marrow and Lung Involvement Treated Successfully with Oral Etoposide. ( 29881869 )
2018
19
Subcutaneous Panniculitis-Like T-cell Lymphoma in Dogs: Morphologic and Immunohistochemical Classification. ( 30071780 )
2018
20
Mycophenolate Mofetil for the Treatment of Subcutaneous Panniculitis-Like T-Cell Lymphoma: Case Report and Review of the Literature. ( 30077696 )
2018
21
Germline HAVCR2 mutations altering TIM-3 characterize subcutaneous panniculitis-like T cell lymphomas with hemophagocytic lymphohistiocytic syndrome. ( 30374066 )
2018
22
Recurrent mutations in epigenetic modifiers and the PI3K/AKT/mTOR pathway in subcutaneous panniculitis-like T-cell lymphoma. ( 28294301 )
2018
23
CCR4 and CCR5 expression in a case of subcutaneous panniculitis-like T-cell lymphoma. ( 28721932 )
2017
24
Immunohistochemistry reveals an increased proportion of MYC-positive cells in subcutaneous panniculitis-like T-cell lymphoma compared with lupus panniculitis. ( 28800143 )
2017
25
Relapsed subcutaneous panniculitis-like T cell lymphoma: role of haploidentical hematopoietic stem cell transplant. ( 28879427 )
2017
26
Subcutaneous Panniculitis-Like T-Cell Lymphoma Presenting as a Local Inflammation of a Thigh in an 8-Month-Old Child. ( 29085769 )
2017
27
Subcutaneous Panniculitis-like T-cell Lymphoma: Immunosuppressive Drugs Induce Better Response than Polychemotherapy. ( 27722764 )
2017
28
Subcutaneous panniculitis-like T-cell lymphoma with haemophagocytic syndrome during tocilizumab therapy for juvenile idiopathic arthritis. ( 28079502 )
2017
29
Clinical characteristics, differential diagnosis, and treatment outcome of subcutaneous panniculitis-like T-cell lymphoma: a literature review of published Japanese cases. ( 28120776 )
2017
30
Subcutaneous panniculitis-like T-cell lymphoma. ( 28127142 )
2017
31
Remission of subcutaneous panniculitis-like T-cell lymphoma in a pregnant woman after treatment with oral corticosteroids as monotherapy. ( 28361107 )
2017
32
Clinical and histopathologic overlap between subcutaneous panniculitis-like T-cell lymphoma with systemic features and small cell CD8+ ALK+ systemic anaplastic large cell lymphoma with cutaneous involvement. ( 27060878 )
2016
33
Hemophagocytic lymphohistiocytosis associated with subcutaneous panniculitis-like T-cell lymphoma. ( 27879081 )
2016
34
Useful Parameters for Distinguishing Subcutaneous Panniculitis-like T-Cell Lymphoma From Lupus Erythematosus Panniculitis. ( 26796503 )
2016
35
Subcutaneous panniculitis-like T-cell lymphoma with macrophage activation syndrome treated by cyclosporine and prednisolone. ( 27990393 )
2016
36
Subcutaneous Panniculitis-Like T-Cell Lymphoma with Hemophagocytic Syndrome in a Child. ( 26764131 )
2016
37
Kikuchi-Fujimoto Disease Associated With Subcutaneous Panniculitis-Like T-Cell Lymphoma. ( 26844615 )
2016
38
Relapsed and refractory subcutaneous panniculitis-like T-cell lymphoma with excellent response to cyclosporine: a case report and literature review. ( 26873089 )
2016
39
Subcutaneous Panniculitis-like T Cell Lymphoma Mimicking Early-Onset Nodular Panniculitis. ( 27342380 )
2016
40
Long-term remission of subcutaneous panniculitis-like T-cell lymphoma with central nervous system involvement: A case report. ( 27347188 )
2016
41
Survival of Subcutaneous Panniculitis-Like T-Cell Lymphoma and Peripheral T-Cell Lymphoma Not Otherwise Specified: A Propensity-Matched Analysis of the Surveillance, Epidemiology, and End Results Database. ( 27349764 )
2016
42
Subcutaneous Panniculitis-Like T-Cell Lymphoma: A Rare Tumour. ( 27437288 )
2016
43
Case report of subcutaneous panniculitis-like T-cell lymphoma complicated by eyelid swelling. ( 27440138 )
2016
44
Systemic T cell lymphoma mimicking subcutaneous panniculitis-like T-cell lymphoma. ( 27444745 )
2016
45
Pediatric subcutaneous panniculitis-like T-cell lymphoma presenting as paraparesis: An unreported manifestation. ( 27625250 )
2016
46
Fluorine-18 fluorodeoxyglucose positron emission tomography/computed tomography evaluation of subcutaneous panniculitis-like T cell lymphoma and treatment response. ( 27672640 )
2016
47
Atypical lymphocytic lobular panniculitis: an overlap condition with features of subcutaneous panniculitis-like T-cell lymphoma and lupus profundus. ( 27807018 )
2016
48
CCR5 and CXCR3 expression in a case of subcutaneous panniculitis-like T-cell lymphoma. ( 26301865 )
2016
49
Hematopoietic stem cell transplantation for primary cutaneous γδ T-cell lymphoma and refractory subcutaneous panniculitis-like T-cell lymphoma. ( 25981001 )
2015
50
Fever and generalized erythema caused by subcutaneous panniculitis-like T-cell lymphoma. ( 26621148 )
2015

Variations for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

ClinVar genetic disease variations for T-Cell Lymphoma, Subcutaneous Panniculitis-Like:

6
# Gene Variation Type Significance SNP ID Assembly Location
1 HAVCR2 NM_032782.5(HAVCR2): c.245A> G (p.Tyr82Cys) single nucleotide variant risk factor GRCh38 Chromosome 5, 157106776: 157106776
2 HAVCR2 NM_032782.5(HAVCR2): c.245A> G (p.Tyr82Cys) single nucleotide variant risk factor GRCh37 Chromosome 5, 156533787: 156533787
3 HAVCR2 NM_032782.5(HAVCR2): c.291A> G (p.Ile97Met) single nucleotide variant risk factor GRCh37 Chromosome 5, 156533741: 156533741
4 HAVCR2 NM_032782.5(HAVCR2): c.291A> G (p.Ile97Met) single nucleotide variant risk factor GRCh38 Chromosome 5, 157106730: 157106730
5 HAVCR2 NM_032782.5(HAVCR2): c.302C> T (p.Thr101Ile) single nucleotide variant risk factor GRCh37 Chromosome 5, 156533730: 156533730
6 HAVCR2 NM_032782.5(HAVCR2): c.302C> T (p.Thr101Ile) single nucleotide variant risk factor GRCh38 Chromosome 5, 157106719: 157106719

Expression for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

Search GEO for disease gene expression data for T-Cell Lymphoma, Subcutaneous Panniculitis-Like.

Pathways for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

GO Terms for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

Sources for T-Cell Lymphoma, Subcutaneous Panniculitis-Like

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
Content
Loading form....